Ukuthi yini i-Pulmonary Atresia ene-Ventricular Septal Defect? Izimpawu, Izimbangela, kanye Nokwelashwa

I-Pulmonary atresia ene-ventricular septal defect iyinkinga enkulu yenhliziyo abantwana abazalwa nayo, lapho i-pulmonary valve ingakhi kahle khona futhi kukhona umgodi phakathi kwamagumbi apompa amakhulu enhliziyo. Lokhu kusho ukuthi igazi elingenayo i-oksijini alikwazi ukugeleza ngokujwayelekile kusuka enhliziyweni kuya emaphashini ukuze lithole i-oksijeni.

Cabanga ngenhliziyo yakho njengoba inamagumbi amane anamasango phakathi kwawo. Kule meko, elinye lala masango (i-pulmonary valve) livaliwe ngokuphelele, futhi kukhona ukuvulwa odongeni phakathi kwamagumbi amabili okungafanele kube khona. Lokhu kudala isimo esiyinkimbinkimbi lapho inhliziyo yengane yakho kufanele isebenze kanzima kakhulu ukuze ithole i-oksijeni emzimbeni.

Ukuthi yini i-Pulmonary Atresia ene-Ventricular Septal Defect?

I-Pulmonary atresia ene-ventricular septal defect (PA-VSD) kwenzeka lapho izinkinga zenhliziyo ezimbili zenzeka ndawonye kusukela ekuzalweni. I-pulmonary valve, okufanele ivuleke ukuze ivumele igazi ligeleze kusuka enhliziyweni kuya emaphashini, ivaliwe ngokuphelele noma ayikho.

Ngesikhathi esifanayo, kukhona umgodi odongeni (i-septum) ohlukanisa amagumbi amabili aphansi enhliziyo (ama-ventricles). Lo mhlanganiso wenza kube nzima kakhulu ukuthi igazi lifike emaphashini ukuze lithole i-oksijeni.

Umkhafa wengane yakho uzama ukubuyisela ngokuthuthukisa imithambo yegazi ehlukile ebizwa ngokuthi i-collateral circulation. Lezi zindlela ezengeziwe zisiza igazi elithile lifike emaphashini, kodwa azikho ngempumelelo njengendlela evamile.

Le meko ithonya cishe 1 kuwo wonke ama-10,000 abantwana abazalwa. Nakuba kuzwakala kukhulu, ukuthuthuka ekukhanda kwenhliziyo yabantwana kwenze ukwelashwa kuphumelela kakhulu kunakuqala.

Ziyini Izimpawu ze-Pulmonary Atresia ene-Ventricular Septal Defect?

Izimpawu ze-PA-VSD zivame ukuvela ezinsukwini ezimbalwa zokuqala noma emavikeni okuqala okuphila kwengane yakho. Ungase uqaphele ukuthi isikhumba, izindebe, noma iinena zengane yakho ziyaphenduka zibomvu, ikakhulukazi ngesikhathi ikhala noma iyancelisa.

Nazi izimpawu ezibalulekile okufanele uzibheke:

• Ukuphenduka kombala oluhlaza wesikhumba, izindebe, noma iinena (i-cyanosis)
• Ubunzima bokuqeda umoya noma ukuqeda umoya ngokushesha
• Ukudla okubi noma ukuqeda amandla ngokushesha ngesikhathi ukudla
• Ukwehluleka ukuzuza isisindo ngokujwayelekile
• Ukuvuvukala okukhulu noma ukukhathazeka
• Ukuqina kwamafingo nezinzwane (imiphetho yeminwe iphenduka iyingqimba futhi ikhulu)
• Ukuhlanza noma iziqephu lapho ingane yakho iphenduka ihlala phansi

Abanye abantwana bangase bathuthukise izinkinga ezingathi sína njengokwehluleka kwenhliziyo, lapho inhliziyo ihluleka ukupompa igazi ngempumelelo. Kwezinye izimo ezingavamile, abantwana bangase bahlangabezane ne-brain abscesses noma i-strokes ngenxa ye-blood clots.

Ubukhulu bezimpawu bungase buhluke kakhulu kuye ngokuthi igazi elingakanani elikwazi ukufika emaphashini ngalezo zindlela ezihlukile esizikhulumile ngaphambili.

Ziyini Izinhlobo ze-Pulmonary Atresia ene-Ventricular Septal Defect?

Odokotela bavame ukuhlukanisa i-PA-VSD ngokusekelwe ekutheni igazi lifika kanjani emaphashini kanye nesakhiwo esithile senhliziyo yengane ngayinye. Umehluko oyinhloko uwukuthi ingane yakho inokugeleza kwegazi okwanele emaphashini noma cha.

Uhlobo 1 luhilela abantwana abanemithambo yegazi enhle ye-collateral ethuthukiswe ngokwemvelo ukuhlinzeka amaphashini. Lezi zindlela ezihlukile zingakunikeza ukugeleza kwegazi okuhle, nakuba kungekhona okuningi njengoba inhliziyo evamile ingakunikeza.

Uhlobo 2 luhilela abantwana abanokugeleza okubi noma okunganele kwe-collateral. Laba bantwana bavame ukudinga ukungenelela okusheshayo ngoba amaphashini abo awutholi ukugeleza kwegazi okwanele.

Kukhona futhi ukuhlukaniswa okuqondene nokuthi ama-pulmonary arteries amakhulu (imithambo emikhulu ethwala igazi kuwo wonke amaphashini) athuthukile kahle noma mancane. Abantwana abanemithambo ye-pulmonary arteries emikhulu, ethuthukisiwe kahle bavame ukuba nemiphumela emihle.

I-cardiologist yengane yakho izonquma ukuthi yiluphi uhlobo olukhona ngokuhlolwa okuqondile kwezithombe, okusisiza ukuqondisa uhlelo lokwelapha.

Yini ebangela i-Pulmonary Atresia ene-Ventricular Septal Defect?

I-PA-VSD ithuthuka phakathi kwamasonto angu-8 okuqala okukhulelwa lapho inhliziyo yengane yakho yakhiwa. Imbangela eqondile ayiqondakali ngokuphelele, kodwa kubonakala sengathi iyinhlanganisela yezinto zofuzo nezemvelo.

Eziningi zezimo zenzeka ngokungahleliwe ngaphandle kwesizathu esicacile. Izakhiwo zenhliziyo yengane yakho azithuthuki ngokujwayelekile phakathi kwalezo zinsuku ezibalulekile zokuqala lapho amalungu akhiwa khona.

Nazi ezinye izinto ezingase zikhulise ingozi:

• Izifo zofuzo njenge-DiGeorge syndrome noma i-CHARGE syndrome
• Umlando womndeni wezinkinga zenhliziyo ezingokuzalwa
• I-diabetes yomzali ngesikhathi sokukhulelwa
• Imithi ethile ethathwe ngesikhathi sokukhulelwa kwasekuqaleni
• Ukutheleleka komzali njenge-rubella ngesikhathi sokukhulelwa
• Ukusetshenziswa kotshwala ngumzali ngesikhathi sokukhulelwa
• Ubudala obukhulu bomzali (ngaphezu kweminyaka engu-35)

Kwezinye izimo ezingavamile, i-PA-VSD ingaba ingxenye yesifo esikhulu sokuzalwa esithinta izinhlelo eziningi zomzimba. Noma kunjalo, abantwana abaningi abale meko bayinayo njengesiphukuphuku senhliziyo esiyodwa.

Kubalulekile ukwazi ukuthi akukho lutho olukwenzile noma awukwenzanga ngesikhathi sokukhulelwa okubangele le meko. Izinkinga zenhliziyo njenge-PA-VSD zenzeka ngesikhathi sokukhulelwa kwasekuqaleni, ngokuvamile ngaphambi kokuba wazi ukuthi ukhulelwe.

Nini Okufanele Ubone Udokotela nge-Pulmonary Atresia ene-Ventricular Septal Defect?

I-PA-VSD ivame ukuxilongwa ngesikhathi sokukhulelwa ngokuhlolwa okujwayelekile kwe-ultrasound, ngokuvamile cishe emavikini angu-18-22. Uma kukhona ukungabaza, udokotela wakho uzokukhipha i-ultrasound ekhethekile ebizwa ngokuthi i-fetal echocardiogram ukuze uthole izithombe ezinemininingwane yenhliziyo yengane yakho.

Ngemva kokuzalwa, ukuxilongwa kuhilela ukuhlolwa okuningi ukuqonda isakhiwo esithile senhliziyo yengane yakho. Umbala wesikhumba sengane yakho, izindlela zokuphefumula, nobunzima bokudla ngokuvamile kunikeza izinkomba zokuqala zokuthi kukhona okuthile okudinga ukunakekelwa.

Inqubo yokuxilongwa ngokuvamile ihlanganisa:

1. Ukuhlolwa komzimba nokulalela inhliziyo
2. I-X-ray yesifuba ukubona usayizi wenhliziyo nokugeleza kwegazi emaphashini
3. I-Echocardiogram (i-ultrasound yenhliziyo) yesakhiwo esinemininingwane yenhliziyo
4. I-Electrocardiogram (EKG) ukuhlola izinga lenhliziyo
5. I-Pulse oximetry ukuqapha amazinga e-oksijeni
6. I-Cardiac catheterization yokulinganisa okuqondile
7. I-MRI noma i-CT scan yezithombe ezinemininingwane

I-Cardiac catheterization ibaluleke kakhulu ngoba ivumela odokotela ukuba babone ngokuqondile ukuthi igazi ligeleza kanjani enhliziyweni yengane yakho futhi balinganise ukucindezela emagumbini ahlukene. Lolu lwazi lubalulekile ekucwaningeni ukwelashwa.

I-cardiologist yengane yakho izophinde ihlole noma yiziphi izimo zofuzo ezihambisanayo noma ezinye izinkinga zenhliziyo ezingase zibe khona. Lokhu kuhlolwa okuhleliwe kusiza ukwakha uhlelo lokwelapha oluphumelelayo sesimo esithile sengane yakho.

Yini Ukwelashwa kwe-Pulmonary Atresia ene-Ventricular Septal Defect?

Ukuphathwa kwe-PA-VSD cishe kuhlale kuhilela ukuhlinzwa, kodwa isikhathi nendlela kuya ngesimo esithile sengane yakho kanye nezimpawu. Umgomo ukusisiza igazi eliningi lifike emaphashini futhi kunciphisa umsebenzi wenhliziyo yengane yakho.

Abantwana abaningi badinga ukuhlinzwa kwabo kokuqala phakathi nezinyanga ezimbalwa zokuqala zokuphila. Le nqubo yokuqala, evame ukubizwa ngokuthi i-Blalock-Taussig shunt, yakha indlela entsha yegazi ukuze lifike emaphashini usebenzisa umthamo omncane.

Uhlelo lokwelapha oluphelele ngokuvamile luhilela ukuhlinzwa okuningi eminyakeni eminingana:

1. Ukuhlinzwa kokuqala kwe-shunt (ngokuvamile ezinyangeni zokuqala zokuphila)
2. Ukuhlinzwa okuphakathi ukuthuthukisa ukugeleza kwegazi emaphashini (cishe ezinyangeni ezingu-4-6)
3. Ukuhlinzwa kokulungisa okuphelele (ngokuvamile phakathi kwezinyanga ezingu-6 kuya kweminyaka engu-2)
4. Ukuhlinzwa okwengeziwe okungenzeka njengoba ingane yakho ikhula

Phakathi kokuhinzwa, ingane yakho izodinga imithi ukuze isize inhliziyo isebenze ngempumelelo futhi ivimbele izinkinga. Lezi zingase zihlanganise i-diuretics ukunciphisa ukwakheka kwamanzi kanye nemithi yokwenza ukupompa kwenhliziyo kuqine.

Abanye abantwana bangase badinge izinqubo ezengeziwe njenge-balloon angioplasty ukuvula imithambo yegazi ecindezelekile noma ukufakwa kwesixhobo ukuvala ukuxhumana okungafuneki phakathi kwemithambo yegazi.

Iqembu lokuhlinzwa lizohlangana nawe ukuze lilinganise isikhathi senqubo ngayinye ngokufanele, sicabangela ukukhula kwengane yakho, izimpawu, kanye nempilo yonke. Ukuhlinzwa kwenhliziyo yabantwana okwamanje kunesilinganiso esihle sempumelelo yokulungisa i-PA-VSD.

Ukunakekela Ekhaya Ngesikhathi se-Pulmonary Atresia ene-Ventricular Septal Defect?

Ukunakekela ingane ene-PA-VSD ekhaya kudinga ukunakekelwa okukhethekile, kodwa imindeni eminingi ihlala ilungisa kahle ngesiqondiso esifanele. Ukugxila kwakho okuyinhloko kuzoba ukulandelela izimpawu zengane yakho nokuqinisekisa ukuthi ithola ukudla okwanele nokuphumula.

Ukudla kungase kube inselele ngoba abantwana abane-PA-VSD bavame ukuqeda amandla kalula. Nikeza ukudla okuncane, okuvamile futhi cabanga ngamafomu aphezulu e-calorie uma udokotela wakho ekukhuthaza ukuze usize ekuzuzeni isisindo.

Nazi izici ezibalulekile zokunakekela ekhaya:

• Landelela umbala wesikhumba, ikakhulukazi ezindebeni naseenena
• Bheka izinguquko ezindleleni zokuphefumula
• Landelela ukuzuza isisindo kanye nezindlela zokudla
• Nikeza imithi ngokuqondile njengoba kulotshiwe
• Vimbela ukutheleleka ngokuhlanzeka okuhle
• Khawukhawuleli ukubonakala kubantu abagulayo uma kungenzeka
• Gcina izikhathi zokulala ezinemigomo
• Landela imikhawulo yemisebenzi njengoba kunconywe

Gcina irekhodi lasemuseni lezimo zengane yakho, amazinga okudla, nanoma yiziphi izinguquko ezinkulu. Lolu lwazi lusiza iqembu lakho lezokwelapha ukwenza izinqumo ezisekelwe ekuqondeni ngesikhathi sokwelapha kanye nokulungiswa.

Dala indawo ethule, enesekelo ekhaya. Abantwana abanezimo zenhliziyo bavame ukwenza kangcono ngezindlela ezinemigomo kanye nokucindezeleka okuncane. Unganqikazi ukucela usizo lomndeni nabangane emisebenzini yansuku zonke ukuze ukwazi ukugxila ekunakekeleni kwengane yakho.

Kufanele Uzilungiselele Kanjani Ukuya Kulodokotela?

Ukulungiselela ukuya kudoctor we-cardiology kusiza ukuqinisekisa ukuthi uthola okuningi kunoma yiluphi uvakatshelo futhi ungakhohlwa imibuzo noma ukukhathazeka okubalulekile. Gcina uhlu oluqhubekayo lokuqaphela ngesimo sengane yakho phakathi kokuvakatshelwa.

Letha irekhodi lezokwelapha eliphelele lengane yakho, kuhlanganise noma yiziphi izivivinyo zamuva noma izinqubo ezivela kwabanye odokotela. Uma ube ugcina idayari yezimpawu, letha leyo ngoba inikeza ulwazi oluyigugu ngezindlela kanye nezinqubo.

Nansi okumele ukulungiselele ngaphambi kokuvakatshelwa ngakunye:

• Uhlu lwemithi yamanje kanye nezinga
• Imibuzo ngesimo sengane yakho kanye nokuphathwa
• Ukukhathazeka ngezimpawu noma izinguquko zokuziphatha
• Ulwazi mayelana nokudla, ukukhula, kanye nokuthuthuka
• Amakhadi yomshuwalense kanye namaphepha adingekayo
• Ulwazi loxhumano oluphuthumayo
• Izinto zokunethezeka zengane yakho

Bhala phansi imibuzo ebalulekile kusengaphambili, njengokubuza ngemikhawulo yemisebenzi, ukuthi ukuhlinzwa okulandelayo kungase kudingeke nini, noma yiziphi izimpawu ezifuna ukunakekelwa okusheshayo. Ungakhathazeki ngokubuza imibuzo eminingi kakhulu - iqembu lakho lezokwelapha lifuna ukuba ulwazi oluhle.

Cabanga ngokuletha ilungu lomndeni noma umngani ukuze uthole ukwesekwa, ikakhulukazi ngesikhathi sokuvakatshelwa lapho kuzoxoxwa khona izinqumo ezibalulekile. Ukuba nomuntu omunye okhona kungakusiza ukuba ukhumbule ulwazi oluyigugu futhi unikeze ukwesekwa ngokomzwelo.

Yini Eyinhloko Ngokuphathelene ne-Pulmonary Atresia ene-Ventricular Septal Defect?

I-PA-VSD iyinkinga enkulu yenhliziyo kodwa elapheka efuna ukunakekelwa kwezokwelapha okukhethekile kanye nokuhlinzwa okuningi ngokuhamba kwesikhathi. Nakuba ukuxilongwa kungase kuzwakale kukhulu, ukuthuthuka ekukhandeni kwenhliziyo yabantwana kuye kwathuthukisa kakhulu imiphumela yabantwana abale meko.

Abantwana abaningi abane-PA-VSD bangaphila impilo egcwele, esebenzayo ngemva kokwelashwa okuphumelelayo. Umgomo ukusebenzisana eduze neqembu lakho le-cardiology yabantwana nokulandela izincomo zabo ngesikhathi sokwenza ukuhlinzwa kanye nokunakekelwa okuqhubekayo.

Uhambo lwengane yakho luzoba oluhlukile, futhi izinhlelo zokwelapha zilungiselelwe isimo ngasinye. Abanye abantwana bangase badinge izinqubo eziningi kunabanye, kodwa umgomo uhlale ukusisiza ingane yakho ifinyelele ezingeni elihle kakhulu lempilo.

Khumbula ukuthi awuwedwa kulolu hambo. Iqembu lakho lezokwelapha, umndeni, namaqembu wokwesekwa angakunikeza usizo nokuqiniswa okubalulekile. Imindeni eminingi iyathola ukuba kuwusizo ukuxhumana nabanye abaye bahamba kule ndlela ngaphambili.

Imibuzo Evame Ukubuzwa Ngokuphathelene ne-Pulmonary Atresia ene-Ventricular Septal Defect

Ingabe ingane yami ene-PA-VSD ingaphila impilo evamile?

Abantwana abaningi abane-PA-VSD bangaphila impilo egcwele, esebenzayo ngemva kokwelashwa okuphumelelayo. Nakuba bazodinga ukulandelela okuphila konke kwenhliziyo futhi bangase babe nemikhawulo yemisebenzi, abaningi bangase bahlanganyele esikoleni, ezemidlalo, nezinye imisebenzi yabantwana. Umgomo ukusebenzisana neqembu lakho lezokwelapha ukuze uphathe le meko ngokufanele futhi ulandele noma yiziphi izinguquko ngokuhamba kwesikhathi.

Zingaki izihlinzwa ingane yami ezizodinga?

Abantwana abaningi abane-PA-VSD badinga ukuhlinzwa okuyinhloko okungu-2-3 phakathi neminyaka yabo embalwa yokuqala yokuphila, kungenzeka kube nezindlela ezengeziwe njengoba bekhula. Inani eliqondile liya ngesakhiwo esithile sengane yakho kanye nendlela abaphendula ngayo ekwelapheni. Udokotela wakho uzoxoxa ngendlela yokuhlela ukuhlinzwa futhi akusize uqonde ukuthi yini okufanele uyilindele kuzo zonke izigaba.

Ziyini imiphumela yesikhathi eside ye-PA-VSD?

Ngokwelashwa okuphumelelayo, abantwana abaningi abane-PA-VSD bangalindela imiphumela emihle yesikhathi eside. Noma kunjalo, bazodinga ukulandelela okuphila konke kwe-cardiology ukuqapha umsebenzi wenhliziyo nokuqapha izinkinga ezingase zenzeke. Abanye bangase babe nemikhawulo yokuvivinya umzimba noma badinge izinqubo ezengeziwe kamuva ekuphileni, kodwa abaningi baphila impilo evamile ngokunakekelwa okufanele.

Ingabe i-PA-VSD ingavikeleka?

Okwamanje, ayikho indlela yokuvimbela i-PA-VSD njengoba ithuthuka ngesikhathi sokukhulelwa kwasekuqaleni ngenxa yezinto eziningi ezingaphandle kokulawulwa komuntu. Noma kunjalo, ukugcina ukunakekelwa okuhle kokukhulelwa, ukuphatha i-diabetes uma ikhona, ukugwema utshwala nokubhema ngesikhathi sokukhulelwa, kanye nokuthatha izithasiselo ze-folic acid ezimiselwe kungase kusize ekuthuthukiseni impilo yonke yengane.

Ingabe i-PA-VSD izowuthinta kanjani ukukhula nokuthuthuka kwengane yami?

Ngaphambi kokuhlinzwa, abantwana abane-PA-VSD bavame ukuba nokuzuza isisindo esincane futhi bangase bafinyelele ezinkingeni zokuthuthuka kamuva kunokujwayelekile. Ngemva kokwelashwa okuphumelelayo, abantwana abaningi bayabamba ngokukhulu kokukhula nokuthuthuka. Iqembu lakho lezokwelapha lizolandelela lezi zindawo eduze futhi linikeze izinsizakalo zokwesekwa uma kudingeka ukuze usize ingane yakho ifinyelele ikhono layo eliphelele.